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Biliary atresia
An infant with jaundice beyond two weeks of age, a raised direct bilirubin and pale stools needs a rapid work-up for biliary atresia — because the Kasai operation works better the earlier it is done, preferably before 60 days of age.
Why it matters in the exam
Biliary atresia is a progressive inflammatory-fibrotic process that obliterates the extrahepatic bile ducts and, untreated, leads to cirrhosis and death in the first years. It is the common surgical cause of cholestasis in infants and the common indication for liver transplantation in children. The exam shows an infant of 4–8 weeks who looks well and grows normally, with jaundice, dark urine, pale (acholic) stools and an enlarged liver, and asks for the next step and its timing.
Under the NASPGHAN/ESPGHAN guideline, a direct (conjugated) bilirubin above 1.0 mg/dL is abnormal at any age and needs work-up. Ultrasound shows a small, irregular or absent gallbladder and sometimes a "triangular cord sign" at the porta hepatis; a HIDA scan shows normal liver uptake with no excretion into the bowel; liver biopsy shows bile ductular proliferation and bile plugs. The final diagnosis is made by an operative cholangiogram. A high GGT supports the diagnosis. A stool colour card for parents brings detection forward.
The Kasai operation (portoenterostomy) joins a Roux loop of small bowel to the porta hepatis after removing the fibrotic remnant. After surgery the common complication is cholangitis — fever, rising bilirubin and stools turning pale again — treated with intravenous antibiotics. Good bile drainage (bilirubin returning to normal) predicts survival with the native liver; even after a successful Kasai most children will eventually need a transplant. About ten per cent have associated anomalies (a syndrome with polysplenia, a preduodenal portal vein and heterotaxy), which worsen the prognosis.
Key points
- Jaundice beyond two weeks — direct bilirubin.
- Direct above 1.0 mg/dL and pale stools — urgent work-up.
- Final diagnosis by operative cholangiogram.
- Early Kasai, preferably before 60 days.
- After Kasai — cholangitis; many will need a transplant.
Summary table
| Test | Typical finding | Meaning |
|---|---|---|
| Bilirubin | Direct above 1.0 mg/dL, high GGT | Cholestasis — not physiological jaundice |
| Ultrasound | Small or absent gallbladder, triangular cord | Supports atresia |
| HIDA | Liver uptake without bowel excretion | Supportive; bowel excretion excludes it |
| Liver biopsy | Bile ductular proliferation and bile plugs | Distinguishes from neonatal hepatitis |
| Operative cholangiogram | Bile ducts do not fill | Final diagnosis, then Kasai |
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Sources
- NASPGHAN/ESPGHAN Guideline for the Evaluation of Cholestatic Jaundice in Infants, 2017 — Conjugated bilirubin cut-off and work-up
- Holcomb and Ashcraft's Pediatric Surgery, 7th edition (2019) — Biliary Atresia
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-06. The text may contain errors; when in doubt, check the source.